Diagnosis and management of severe congenital protein C deficiency (SCPCD): Communication from the SSC of the ISTH. Academic Article uri icon

Overview

abstract

  • Severe congenital protein C deficiency (SCPCD) is rare and there is currently substantial variation in the management of this condition. A joint project by three Scientific and Standardization Committees of the ISTH: Plasma Coagulation Inhibitors, Pediatric/Neonatal Thrombosis and Hemostasis, and Women's Health Issues in Thrombosis and Hemostasis, was developed to review the current evidence and help guide on diagnosis and management of SCPCD. We provide a summary of the clinical presentations, differential diagnoses, appropriate investigations to confirm the diagnosis, approaches for management of the acute situation, and options for long-term management including subsequent pregnancies. We finally provide a set of recommendations to help in this regard.

publication date

  • May 15, 2022

Research

keywords

  • Disseminated Intravascular Coagulation
  • Protein C Deficiency
  • Thrombosis

Identity

Scopus Document Identifier

  • 85132647530

Digital Object Identifier (DOI)

  • 10.1111/jth.15732

PubMed ID

  • 35570324

Additional Document Info

volume

  • 20

issue

  • 7